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16 Cards in this Set

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clinical features of frontotemporal dementias
progressive language deterioration
personality changes
lobes affected in FTD
frontal and temporal lobes
aka for FTD
tauopathy because all have accumulation of tau deposits
FTD with Parkinsonism and tau
pathology:
cortical atrophy, neuronal loss, gliosis, tau-tangles (4R or 3R+4R); +/- glial inclusions
What are the tauopathies?
FTD with Parkinsonism
Pick dz
Progressive supranuclear palsy
corticobasal degeneration
Pick dz (lobar atrophy) clinical:
early onset behavioral change, personality change (frontal lobe), and language alterations (temporal lobe); mostly sporadic, but some familial with tau mut
Pick dz pathology gross
asymmetric frontal and temporal atrophy (knife-edge); SPARING of posterior two-thirds of superior temporal gyrus; essentially no parietal or occipital involvement; +/1 bilateral caudate and putamen atrophy
Pick dz pathology micro
severe neuronal loss in outer 3 layers of cortex, Pick cells, Pick bodies
what are Pick cells?
Pick cells = swelling of neurons
what are Pick bodies?
Pick bodies = round homogeneous cytoplasmic filamentous inclusions, weak basophilic and strongly silver stained (3R tau)
progressive supranuclear palsy clinical
truncal rigidity, dysequilibrium, speech and ocular disturbance, dementia
progressive supranuclear palsy path
globose tangles of 4R tau in globus pallidus, subthal nucl, subst nigra, periaqueductal grey, dentate
progressive supranuclear palsy genetics
no MAPT (tau) mutation, but some have MAPT polymorphism
Corticobasal degeneration path
motor cortex atrophy; neuronal loss, gliosis, "ballooned neurons" (phosphorylated neurofilaments);
where is tau in corticobasal degeneration?
tau (4R) is in astrocytes (tufted), oligos (coiled bodies), basal gangl neurons, cortical neurons; around astrocytes (astrocytic plaques); threads in grey and white matter; subst nigra involvement
FTD's without tau usually contain what?
ubiquitin inclusions
some with mut on chr 17 progranulin (close to MAPT tau)